Superficial granulomatous pyoderma with ocular involvement

ARTICLE

Auteur(s) : Mieke Dierick¹, Sofie De Lille¹, Ilse Claerhout², Pieter-Paul Schauwvlieghe², Sofie De Schepper¹, Marc Haspeslagh¹, Liesbet Vanquickenborne³, Hilde Beele¹, Lieve Brochez¹

¹Dept of Dermatology, Ghent University Hospital, De Pintelaan 185, 9000 Ghent, Belgium
²Dept of Ophthalmology, Ghent University Hospital, Ghent, Belgium
³Dept of Dermatology, AZ Sint-Lucas, Bruges, Belgium

A 77-year-old woman presented with an ulcer above the lip, which started as a pustule evolving into a rapidly progressive, painful ulceration with an intermittent seropurulent discharge. Examination showed a 2 cm ulceration with a raised inflammatory border and a crusted surface. Biopsy showed an aspecific ulceration with a dense neutrophilic inflammatory infiltrate and some granulomas in the papillary dermis (figure 1A). There were no signs of malignancy. PAS, Grocott, Ziehl-Nielsen and Giemsa stains were negative. Bacterial, fungal and mycobacterial cultures of the discharge and tissue remained negative. Blood analysis showed signs of inflammation with an increased sedimentation rate. The diagnosis of superficial granulomatous pyoderma (SGP) was suggested and treatment with oral methylprednisolone 16 mg/d was started.

After three weeks the patient presented with multiple new lesions on the face and scalp and an ocular lesion (figure 1B). She mentioned blurred vision and a painful sensation in the eye. The patient was hospitalised for extensive internal screening (chest radiography, abdominal and lymph node ultrasonography, mammography, bone marrow punction, gastro-intestinal and gynaecologic examination). These investigations were within normal limits and there was no evidence for any associated disease or malignancy.

Ophthalmologic examination showed seriously hampered vision in the left eye. Biomicroscopy revealed a severely inflamed eye, with generalized corneal edema and massive corneal melting. On the sclera, two nodular lesions were seen with scleral melting and necrosis between them (figure 1C). These findings were suggestive for sclerokeratitis in the context of pyoderma gangrenosum (PG).

Methylprednisolone was increased to 64 mg/d and associated with broad spectrum antibiotics. This resulted in a spectacular improvement of the cutaneous and ocular lesions and visual acuity. Systemic corticotherapy was gradually tapered and stopped after 4 months.

After a follow-up of nine months the patient presented with a recurrence and methylprednisolone was restarted. For the moment (follow-up period of 3 years) there is residual scarring above the lip and there is a permanent nasal scleromalacia and corneal thinning. There are no active lesions and no associated disease has been detected to date.

PG belongs to the neutrophilic dermatoses. Its aetiology is unknown, although an autoimmune basis is suspected. SGP is the vegetative variant [1]. It is characterized by a sterile, slowly progressive, usually painless ulcer with raised borders. It is usually less inflamed, has a more chronic course and is associated less with systemic disease than classical PG. There is often a pathergy phenomenon [1, 2].

SGP is a diagnosis by exclusion, based on clinicopathological features. Differential diagnoses primarily include mycobacterial, fungal and amoebic infections, foreign-body granulomas and ulcerative sarcoidosis [1, 2]. Histological examination shows a neutrophilic infiltrate with some granulomas in the dermis, typically organised in three layers: an innermost zone with neutrophils, a surrounding layer with histiocytes and giant cells and an outer layer containing a mixed inflammatory infiltrate with plasma cells and eosinophils. Cultures of pus and tissue are negative [1].

In our case there was also sclerokeratitis. Ocular involvement, especially a peripheral ulcerative keratitis, is reported in classical PG in a number of case reports. Only one case report describes SPG with severe episcleritis and another with palpebral lesions [3, 5, 6].

SGP has a chronic course with frequent relapses. In contrast to classical PG, it usually responds to less aggressive therapy and some lesions may resolve spontaneously. Some cases respond to topical or intralesional steroids or to systemic anti-inflammatory treatment (tetracyclines, sulphapyridine, dapsone). In some cases systemic steroids are required. Cyclosporine, isotretinoin and intravenous immunoglobulines have been reported as alternatives [1, 3, 4].

Acknowledgements

References

2 Lichter MD, Welykyj SE, Gradini R, Solomon LM. Superficial granulomatous pyoderma. Int J Dermatol 1991; 30: 418-21.

3 Goettmann S, Saiag P, Guillaume JC. Pyoderma gangrenosum superficial avec histologie pseudo-tuberculoïde et atteinte oculaire. Ann Dermatol Venereol 1989; 116: 831-2.

4 Lachapelle J-M, Marot L, Jablonska S. Superficial granulomatous pyoderma gangrenosum of the face, successfully treated by ciclosporine: a long-term follow-up. Dermatology 2001; 202: 155-7.

5 Ayyala RS, Armstrong S. Corneal melting and scleromalacia perforans in a patient with pyoderma gangrenosum and acute myeloid leukemia. Ophtalmic Surg Lasers 1998; 29: 328-31.

6 Rose GE, Barnes EA, Uddin JM. Pyoderma gangrenosum of the ocular adnexa. A rare condition with characteristic clinical appearances. Ophthalmology 2003; 110: 801-5.

* Drs De Lille and Dierick contributed equally to this article and share first authorship